Hypocomplementaemia as an immunological marker of morbidity and mortality in patients with primary Sjogren's syndrome

Abstract

Hypocomplementaemia is closely associated with systemic expression and adverse outcomes (lymphoma development and death) in patients with primary SS. Our results support the inclusion of complement determination at diagnosis as a predictor of the outcome of patients with primary SS and its routine determination in the clinical follow-up.

Mixed cryoglobulinemia

“…As observed for HCV infection, the prevalence of other virus-related MC seems to show a variable prevalence among patient's series from different geographical areas [86]. Moreover, a number of clinicoepidemiological studies revealed a heterogeneous distribution of different HCV-related extra hepatic manifestations, including some autoimmune disorders such as 'primary' Sjogren's syndrome [87][88][89][90][91][92][93][94][95][96][97][98].…”

“…Cryoglobulinemic syndrome may share a number of etiopathogenetic events and clinical features with both autoimmune diseases such as autoimmune hepatitis, Sjö-gren's syndrome, and polyarthritis, and B-cell lymphomas [3,11,12,25,28,[85][86][87][88][89][90][91][92][93][94][95][96][97][98][99][100]. Therefore, a differential diagnosis should be carefully done in all patients with MC syndrome (Fig.…”

“…Cryoglobulinemic syndrome can represent a crossroads between some autoimmune diseases (autoimmune hepatitis, Sjögren's syndrome, polyarthritis, glomerulonephritis, thyroiditis, type 2 diabetes, etc.) and malignancies (B-cell lymphomas, hepatocellular carcinoma) [3,11,12,25,28,[87][88][89][90][91][92][93][94][95][96][97][98]. It is possible to observe in Differential diagnosis between mixed cryoglobulinemia and other autoimmune-lymphoproliferative disorders in the setting of HCV infection the same patient a slow progression from mild HCV-associated hepatitis to various extrahepatic manifestations (arthralgias, sicca syndrome, Raynaud's phenomenon, RF positivity, etc.…”

“…MC and pSS may share various symptoms: xerostomia and/or xerophthalmia, arthralgias, • arthritis: differential diagnosis with RA (see Fig. 7) • thyroid involvement: hormones, auto-Ab, neck US, fine-needle aspiration • B-cell lymphoma: clinical monitoring; bone marrow/lymph node biopsies, total body CT scan Abbreviations: F: rheumatoid factor; ANA: antinuclear antibodies; anti-ENA: anti-extractable nuclear antigen antibodies; AMA: antimitochondrial antibodies; ASMA: anti-smooth muscle antibodies; anti-LKM1: anti-liver/kidney microsome type 1 antibodies; ALT: alanine aminotransferase; AP: alkaline phosphatase; US: ultrasonography; CT: computed tomography; EMG: electromyography; RA: rheumatoid arthritis; SS: Sjögren's syndrome purpura, RF and serum cryoglobulins, and the possible complication with B-cell lymphoma [3,11,12,25,87,[93][94][95][96][97][98]. However, a careful patient clinical assessment is usually sufficient for a correct diagnosis in the large majority of cases by considering some important findings: histopathological alterations of salivary glands and specific autoantibody pattern (anti-RoSSA/LaSSB) of pSS are rarely found in MC patients; conversely, HCV infection, cutaneous leukocytoclastic vasculitis, and visceral organ involvement (renal, liver) are seldom recorded in primary Sjögren's syndrome (Fig.…”

“…In rare cases in which the differential diagnosis may result very difficult, particularly in HCV-negative individuals, it might be correct to classify the disorder as overlap syndrome. However, patients with MC/pSS overlap syndrome are often characterized by more severe clinico-prognostic evolution; they show a significant low rate of anti-RoSSA/LaSSB along with a high prevalence of mixed cryoglobulinemia, hypocomplementemia, systemic autoimmune manifestations, and complicating lymphomas (94,97,98). In the clinical practice, this particular condition could be better regarded as vasculitic syndrome with relevant implications on the patient monitoring and treatment [11,24,25].…”